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Respiratory > Interstitial lung disease > Flashcards

Interstitial lung disease Flashcards

1
Q

What is an interstitial lung disease?

A

Any disease effecting the lung interstitium- the alvioli and terminal bronchi

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2
Q

What type of pattern would you see in spirometry for a patient with interstitial lung disease?

A

Restrictive pattern because gas exchange is effected

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3
Q

What are the common symptoms of ILD?

A

Breathlessness and DRY cough

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4
Q

ILD can be acute, episodic or chronic. What are the 4 types of ILD?

A

1) ILD of known cause
2) Idiopathic interstitial pneumonia (IIP)
3) Granulomatous ILD
4) Other forms of ILD

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5
Q

What are the causes of ‘known cause ILD’?

A

Rheumatoid arthritis, collagen vascular disease, drugs and association with other diseases

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6
Q

What are the 2 types of idiopathic interstitial pneumonia?

A

Idiopathic pulmonary fibrosis (IPF) and not IPF

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7
Q

What causes granulomatous ILD?

A

Sarcoidosis and hypersensitivity pneumonitis

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8
Q

What is sarcoidosis?

A

A type 4 hypersensitivity reaction of unknown causes that causes granulomatous ILD

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9
Q

Sarcoidosis can be a systemic disease. What are the most common system involvements?

A

Lung and hyler lymph nodes

joints, liver, kidneys, brain, nerves and heart

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10
Q

What type of granuloma forms in sarcoidosis?

A

Non caseating granuloma

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11
Q

Sarcoidosis is more common in smokers. True or false?

A

False

Sacoidosis is more common in non smokers

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12
Q

What are the symptoms of acute sarcoidosis?

A 
Erythema nodosum
Bilateral hyler lymphadenopathy
Arthritis 
Uveitis and parotitis (inflammation of the middle layer of eye and parotid gland)
Fever and malaise
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13
Q

What are the symptoms of chronic sarcoidosis?

A 
Lung infiltrates- alviolitis
Skin infiltrates
Peripheral lymphadenopathy
Hypercalemia 
Other organ involvement
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14
Q

What are the differential diagnosis for sarcoidosis?

A

TB, Lymphoma, Carcinoma and fungal infection

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15
Q

What investigations should you do if you suspect sarcoidosis?

A

CXR- specifically bilateral hyler lymph nodes
CT scan- peripheral nodular infiltrates
Tissue biopsy- non caseating granuloma (multinucleated giant cells)
Pulmonary function- spirometry and TLCO
Bloods: ACE levels- monitoring not diagnosis (test for granuloma)
Raised calcium
Increased inflammatory markers

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16
Q

It is common to have a relapse of sarcoidosis. True or false?

A

True- must monitor CXR and pulmonary function for years

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17
Q

WHat is the treatment for acute sarcoidosis?

A

No treatment unless vital organs are involved and then steroids are given. Its self limiting

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18
Q

What is the treatment for chronic sarcoidosis?

A

Oral steroids needed for 1-2 years

If you stop steroids and then get a relapse you may need immunosupressive drugs such as methotrexate or azathioprine.

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19
Q

What are the pulmonary function restrictive signs?

A

Low FEV1 and low FVC
High/normal FEV1:FVC ratio
Low TLCO

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20
Q

What is hypersensitivity pneumonitis?

A

Type 3 hypersensitivity reaction where immune complexes are deposited in the lungs leading to alviolitis

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21
Q

What is the aetiology of hypersensitivity pneumonitis?

A

Mostly occupational: farmers lung, bird fancier’s lun, cheese workers lung.
Idiopathic in 30% of cases

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22
Q

Can hypersensitivity pneumonitis be acute or chronic?

A

Yes- depends on the length of exposure.
Little and often- chronic
All at once- acute

23
Q

What are the signs of acute hypersensitivity pneumonitis?

A

Cough, breathlessness, fever, maialgia
Symptoms occur several hours after exposure
Crackles and hypoxia

24
Q

What is the treatment for acute hypersensitivity pneumonitis and what would you see on a CXR?

A

Widespread pulmonary infiltrates on CXR

Treatment = oxygen, steroids and antigen avoidance

25
Q

What are the signs of chronic hypersensitivity pneumonitis?

A

Repeated low dose antigen exposure over years
Progressive breathlessness and cough
Crackles in chest

26
Q

What would you expect to see on a CXR and pulmonary function tests of someone with chronic hypersensitivity pneumonitis?

A

CXR = pulmonary fibrosis in the upper zones

Pulmonary function is restrictive

27
Q

What is a diagnosis of chronic hypersensitivity based on?

A

History of exposure and precipitins- measuring the IgG antibody to the antigen in question

28
Q

What is the treatment for chronic hypersensitivity pneumonitis?

A

Remove antigen exposure and oral steroids

29
Q

Is idiopathic pulmonary fibrosis an inflammatory condition and is it more common in smokers?

A

It is not an inflammatory condition but is more common in smokers.

30
Q

What is the characteristics of IPF?

A

Lung is repairing itself when there is nothing to repair => scar tissue
Looks similar to pulmonary fibrosis of known causes

31
Q

Which drugs can cause IPF?

A

Methatrexate, Nitrofuratonin, Ameodarone, Bleomycin, Penicillamine, Busulphan

32
Q

What is the presentation, examination findings and investigations for IPF?

A 
Presentation = progressive breathlessness over several years and dry cough
Examination = Clubbing and bilateral crackles 
Investigations = Pulmonary function (restrictive), CXR (bilateral infiltrates) CT (Reticulondular fibrotic shadowing worse at the base and periphery. Traction bronchiectasis and honey combing)
33
Q

What would you expect to see on a CT scan of someone with IPF?

A

Reticulondular fibrotic shadowing worse at the base and periphery. Traction bronchiectasis and honey combing.
If the presentation is atypical a lung biopsy should be done

34
Q

What are the differential diagnoses of IPF?

A

Occupational disease, connective tissue disease, left ventricular failure, sarcoidosis, hypersensitivity pneumonitis

35
Q

What is the treatment for IPF?

A

Steroids and immunosupressants do NOT change the course of the disease
Pirfenidone and Nitedanib slow down disease progression but are expensive
Oxygen if hypoxic
Lung transplant if under 65

36
Q

Give 4 examples of occupational lung disease

A

Bariosis, Silicosis, Coal workers pneumoconiosis and asbestosis

37
Q

What is simple pneumoconiosis?

A

No patient symptoms or lung function impairment, just an CXR abnormality

38
Q

What is complicated pneumoconiosis?

A

Progressive massive fibrosis

Restrictive pattern and breathlessness

39
Q

What is Caplan’s syndrome?

A

Rheumatoid pneumoconiosis

Patient with rheumatoid arthritis also exposed to coal dust

40
Q

What are the 4 types of pleural disease due to asbestos exposure?
Only one fibre needed

A

1) Benign pleural plaques- asymptomatic
2) Acute asbestos pleuritic- fever, pain, bloody pleural effusion
3) Pleural effusion and diffuse pleural thickening- restrictive inpariment
4) Mesothilioma- fatal within 2 years

41
Q

Can asbestos cause pulmonary fibrosis?

A

Yes if heavy, prolonged exposure. Called Asbestosis.
Diffuse pulmonary fibrosis and restrictive defect.
Asbestos fibres in biopsy and sputum

42
Q

How does asbestos exposure effect the risk of bronchial carcinoma in smokers?

A

Multiplies the risk

43
Q

What is within the pulmonary interstitium?

A

Alveolar ling cells- types 1 and 2 pneumocytes

Thin elastin rich connective tissue containing capillary blood vessels

44
Q

What are the characteristics of early and late stage ILD?

A 
Early = alveolitis- injury with inflammatory cell infiltration 
Late = diffuse fibrosis
45
Q

Give 2 biopsies often used in the diagnosis of ILD?

A

Transbronchial biopsy- forceps used at bronchoscopy

Thoracoscopic biopsy- more invasive but more reliable and generates more tissue

46
Q

What are the pathological changes you may see in IPF?

A

Peripheral and basal fibrosis- honey combing and thickening
Inflammatory component is variable
Terminally, lung structures are replaced by dilated spaces surrounded by fibrous walls

47
Q

What are the pathological changes you may see in hypersensitivity pneumonitis?

A

Inflammatory interstitial expansion
Granulomas with multi nucleated giant cells, infiltration
NO necrosis

48
Q

What are the pathological changes you may see in sarcoidosis?

A

Granulomas with multi nucleated giant cells. NO necrosis and NO destruction of surrounding tissues
If it becomes chronic you will see fibrosis

49
Q

Connective tissue diseases such as lupus and rheumatiods disease can cause ILD. What pathological changes may you see in ILD caused by connective tissue disorders?

A

Interstitial fibrosis
Pleural effusions
Rheumatoid nodules occurring subcutaineously and in deep tissues

50
Q

What are the pathological changes you may see with pneumoconiosis?

A

Nodular scarring and fibrous reaction to mineral dust inhaled

51
Q

Pneumoconiosis is due to the inhalation of mineral dust. What does the disease depend on?

A

1) particle size must be between 1-5 microns
2) Reactivity of the particle
3) Clearance of the particle
4) Host response

52
Q

Which asbestos particle is safer and which is more dangerous?

A

Serpentine (curved) = relatively safe

Amphibole (straight) = highly dangerous

53
Q

How are asbestosis bodies identified at post mortem?

A

By incinerating the lung tissue

Respiratory (26 decks)

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